Retrospective case-analysis of the magnetic resonance imaging characteristics in the brain of patients with Wilson disease

BACKGROUND:Wilson disease (WD)damages liver,brain,kidney,cornea and nervous system severely.It is manifested in four ways:brain,liver,kidney and bone muscle.Whether or not magnetic resonance imagling (MRI)can clearly display the diseased region and range in brain of patient with WD,which provides imageological evidence for clinical practice,is unclear.OBJECTIVE:To observe the charactedstics of MRI of brain in patient with SD,and analyze the correlation of diseased region with clinical symptoms.DESIGN:Retrospective case-analysis.SETTING:Department of Radiology,Second Hospital Affiliated to the General Hospital of Chinese PLA.PARTICIPANTS:Thirty-one patients,including 18 males and 13 females,with WD admitted to the Department of Neurology,Second Hospital Affiliated to the General Hospital of Chinese PLA between January 1999and December 2005 were retrieved.The involved patients presented serum copper oxidase (sCP) activity decreasling and/or caruloplasmin Ievel decreasling and/or urinary copper content increasling;typical extrapyramidal symptoms and/or physical sign;abnormality showed by slit-lamp examination,Kayser-Fleischer rling positive.METHODS:①All the involved patients underwent MRI examination.A GE 1.5T imagling equipment was used.Spin-echo sequence was adopted to perform T2 and T1-weighed image at transverse axis level.Partial cases subjected to head scannling at coronal and/or sagittal level.Gd-DTPA With dosage of 0.1 mmol/kg was the strongest in 4 cases.②MRI characteristics of patients with dliferent clinical symptoms were observed.MAIN OUTCOME MEASURES:MRI detection results of patients with WD and MRI characteristics of patients with different clinical symptoms.RESULTS:Thirty-one patients with WD participated in the result analysis.①Imageological examination results:WD lnvolved many regions in the brain:dorsal caudate putamen(n=28),thalamencephalon(n=25),mesencaphalon(n=25),globus pallidus(n=23),pons(n=21),posterlor limb of intemal capsule(n=16),dentate nucleus(n=16),caudata nucleus(n=15)and cerebral cortex(n=11).MRI presented hypo-intensity signal on T2-weighted image and T1-weighted image or isointensity signal on T1-weighted image in 24 patients,characteristic hypo-intensity signal of globus pallidus in 4 patients,mixed signal of hyper-and hypo-intensity in 2 patients,hypo-intensity signal of globus pallidus,pars anterior pedunculi carebd and pontine tegmentum on T1-weighted image in 1 patient.Pathological changes distdbuted in symmetry and focus of infection mostly presented mottling,lamellar or strip.Different degrses of cerebral cortex atrophy,especially subtentodal cerebellar atrophy,ware found in 20 patients.Four patients subjected to enhancement scannling,but no clear imaqling was found.③MRI characteristics of patients with different symptoms:Abnormal signal of dorsal caudate putamen was found in 28 patients with dystonia,21 patients with dysarthda and 16 patients with bradykinesia;Abnormal signal of mesencaphalon was found in 22 patients with trepidation,among which,18 presented abnormal signal of pons;Abnormal signal of caudate nucleus and lenticular nucleus was found in 15 patients with dysphagia;Abnormal signal of dentate nucleus was found in 16 patients with carebellar ataxia;Different degrees of changes in cerebral atrophy were found in 14 patients with detedoratling memory and dementia.CONCLUSION:MRI can clearly display the characteristics of diseased regions in brain of patient with WD.Diseased regions reflected by MRI have obvious differences in patients with different clinical neurosigns.     

对称性颅脑损伤的临床诊断与治疗(附123例报告)

目的提高治疗对称性颅脑损伤临床疗效。方法总结123例对称性颅脑损伤患者的临床资料,对其临床特点和治疗情况进行回顾性分析。结果预后良好64例(52.0%),中残25例(20.3%),重残8例(6.5%),植物生存4例(3.3%),死亡22例(17.9%)。结论对称性颅脑损伤是一种较为危重的特殊损伤类型,重视其临床特点,严密观察病情变化,正确把握治疗适应证,方可提高疗效。     

LPS信号转导分子TLR4表达与小鼠肝损伤的关系

目的：探讨脂多糖(1ipopolysaccharide，LPS)信号转导分子，TLR4(Toil-like receptor 4,TLR4)与肝脏损伤的关系。方法：小鼠腹腔注射LPS后不同时间点取肝脏组织，HE染色观察病理改变，RT-PCR法检测肝组织，TLR4 mRNA的表达。结果：腹腔注射LPS后24h小鼠肝脏出现明显炎性浸润、坏死，肝组织TLR4 mRNA的表达在注射LPS后6，12h明显抑制，24h则基本恢复。结论：LPS致肝损伤呈时间依从性；LPS信号转导分子TLR4在介导LPS肝损伤中起重要作用。。     

经肝动脉灌注三氧化二砷对肝移植瘤血管的作用

目的 :探讨经肝动脉灌注As2 O3 对肝移植瘤血管的作用 .方法 :采用新西兰大白兔建立 5 0只肝脏Vx 2移植瘤动物模型 ,随机平均分成 5组 .移植瘤术后 2wk ,经肝动脉插管灌注不同浓度As2 O3 ,并以顺铂和生理盐水作对照 ,连续 7d ,6wk末切取肝肿瘤组织 ,观察肿瘤组织微血管有无改变 ,检测移植瘤组织血管内皮生长因子 (VEGF)的表达 .结果 :实验组肿瘤组织毛细血管内皮细胞肿胀、细胞核碎裂、细胞坏死及微血栓形成 ,阴性对照组和阳性对照组无类似改变 .实验组VEGF表达低于对照组 ,差异有显著性 (P <0 .0 1 ) ,而实验组间VEGF表达无差异性 (P =1 .0 0 ) .结论 :经肝动脉灌注As2 O3可减少肿瘤组织的血管生成 ;选择性破坏肝移植瘤组织血管 ,并存在一定剂量效应相关性     

Surgical treatment for carcinoma of the papilla of vater

Eighty of 89 patients who underwent radical resection (resectability 89.9%) for carcinoma of the papilla of Vater between 1976 and 1992 were retrospectively reviewed. Seventy-three patients underwent pancreaticoduodenectomy (PD) and 7 underwent pylorus-preserving pancreaticoduodenectomy (PPPD). The postoperative mortality rate was only 3.8% (3 patients). The 3- and 5-year survival rates were 63.6% and 57.4%, respectively. Important factors influencing long-term survival were Stage (clinical stage = Stage), microscopic lymph node metastasis (n), duodenal wall invasion (d), vascular invasion (v), and the epithelium of origin. Early carcinoma of the papilla of Vater is defined as tumor in which invasion is limited within the papilla of Vater; in particular, carcinomatous invasion is within the muscle of Oddi (d₀) with n₀. PD and/or PPPD with radical lymph node dissection should be performed for carcinoma of the papilla of Vater, as these procedures can be performed with low morbidity and mortality.     

726例小儿急性中毒的特点及预防探讨

[目的]探讨小儿急性中毒的特点及预防方法。[方法]回顾分析5年来共726例小儿急性中毒的一般临床调查资料。[结果]小儿急性中毒近年来明显增多,发病年龄高峰为小于3岁及大于8岁,中毒种类多,医源性中毒、误服和自杀中毒有增多趋势。[结论]预防措施应重在加强对食物卫生及医疗单位尤其是基层医疗机构执业规范的监督管理,并应注意青少年的心理健康教育。     

Michaelis—Menten兼有一级消除动力学模型平均稳态时的一些特性

本文将Rowland等人的二房室模型中的消除过程扩展成米氏兼一级消除过程。据此,推导出达到稳态浓度的时间、血药浓度-时间曲线下面积和生物利用度的公式;进而得到平均清除率和剂量间的关系以及剂量与平均清除率和血浆浓度呈线性关系的结论。     

室间隔缺损患者肺动脉压的多因素分析

本文对４３例经心导管证实的空间隔缺损患者进行了多因素逐步回归分析，发现在２３项临床参数中，对肺动脉压有显著影响的因素，依次为红细胞压积、周围血管征、空间隔缺损大小、心脏杂音强度、心胸比率（ｒ＝０．８０２８），并初步探讨了各因素对肺动脉压的影响机制，旨在能非创伤性地动态了解患者肺动脉压力，为临床工作提供参考依据。     

Cutaneous tumour-like lesions due to poxvirus infection in Chilean flamingos.

Cutaneous tumour-like growths were observed on the face and other areas of the body surface of young Chilean flamingos. In the cells of these lesions, avian pox-specific cytoplasmic inclusion bodies were observed by light microscopy and virus particles were detected under an electron-microscope. It was diagnosed as avian pox.